Cutar fibrosis na tumaki masu tsokanar cuta ( IPF ) wani nau'i ne na cututtukan kwayar cuta wanda ke haifar da dyspnea gaba daya (shortness na numfashi). Mutanen da ke da IPF zasu iya shawo kan tari na busassun da ci gaba, gajiya mai saurin, ko rashin hasara maras kyau - kuma sau da yawa, mutuwar farko.
IPF wani cuta ne wanda ba a sani ba, amma ba a dauka bace. An kiyasta kimanin mutane 15,000 su mutu daga IPF kowace shekara a Amurka.
Yana shafar maza sau da yawa fiye da mata, masu shan taba fiye da wadanda basu taba shan taba ba, kuma yawancin mutane fiye da shekaru 50.
Dalilin IPF bai riga ya yi aiki ba ("idiopathic" na nufin "rashin sananne"), kuma babu magani ga shi. Duk da haka, ana gudanar da bincike mai yawa don fahimtar wannan yanayin, da kuma inganta sana'o'i masu kyau ga IPF . Binciken ga mutanen da ke da IPF sun riga sun inganta sosai a cikin 'yan shekarun nan.
Ana samun sababbin sababbin hanyoyin magance IPF, wasu kuma sun riga sun gwada gwaji. Yana da kyau a farkon don tabbatar da cewa nasara a magani yana daidai ne a kusurwa, amma akwai dalili mafi yawa ga fatawa fiye da lokacin da ya wuce.
Mu fahimtar fahimtar IPF
IPF yana lalacewa ta hanyar fibrosis (tsokafi) na ƙwayar huhu. A cikin IPF, ƙananan sel na alveoli (jakar iska) suna maye gurbin da sauƙi, ƙwayoyin fibrotic waɗanda basu iya yin musayar gas.
A sakamakon haka, babban aikin aikin kayan musayar huhu, yana barin oxygen daga iska don shiga jini, da kuma carbon dioxide don barin jini-an rushe shi. Hanyoyin da ke tattare da hankali don samun isasshen iskar oxygen a cikin jini shine abin da ya sa mafi yawan alamun bayyanar IPF.
Shekaru da yawa, ka'idar aiki game da hanyar IPF ta kasance daya ne akan ƙonewa.
Wato, an yi tunanin cewa wani abu ya sa kumburi da jikin mutum ya ƙone, wanda ya haifar da matsanancin matsala. Saboda haka an yi amfani da magunguna na farko na IPF don karewa ko kuma rage jinkirin aikin mai kumburi. Irin wannan jiyya sun haɗa da steroid , methotrexate , da cyclosporine . Ga mafi yawancin, wa] annan jiyya ba su da tasiri kawai (idan a kowane lokaci), kuma suna da tasiri mai mahimmanci.
Yayinda yake bayyana dalilin da ake kira IPF, masu bincike a yau sun janye hankalin su daga wani tsari mai mahimmanci na flammatory, kuma ga abin da ake zaton yanzu shine tsari na warkar da cutar huhu a cikin mutane da wannan yanayin. Wato, ainihin matsalar da ke haifar da IPF bazai zama mummunan lalacewar nama ba, amma wariyar warkarwa daga (yiwu ko al'ada) lalacewar nama. Tare da wannan warkarwa ta haɗari, yawancin fibrosis yana faruwa, wanda zai haifar da lalacewa mai tsabta.
Maganin al'ada na huhu ya juya ya zama tsari mai ban mamaki, wanda ya haɗa da haɗuwa da nau'o'in kwayoyin halitta da kuma yawan abubuwan ci gaba, cytokines da sauran kwayoyin. Mafi yawan fibrosis a cikin IPF yanzu anyi tunanin cewa za'a danganta da rashin daidaituwa a tsakanin wadannan abubuwa daban-daban a lokacin warkarwa.
A hakikanin gaskiya, an gano wasu ƙwayoyin cytokine da kuma abubuwan haɓaka da yawa da ake tsammani suna taka muhimmiyar rawa wajen raya fibrosis na huhu.
Wadannan kwayoyin yanzu sune makasudin bincike mai zurfi, kuma ana amfani da kwayoyi masu yawa kuma an gwada su cikin kokarin sake dawo da tsarin al'ada a cikin mutanen da ke da IPF. Ya zuwa yanzu, wannan bincike ya haifar da wasu nasara da dama da yawa-amma nasarorin sun kasance masu ƙarfafa sosai, har ma da rashin gamsuwa sun ci gaba da iliminmu game da IPF.
Successes So Far
A shekarar 2014, FDA ta amince da sababbin sababbin kwayoyi don maganin IPF , nintendanib (Ofev) da kuma pirfenidone (Esbriet).
Wadannan kwayoyi sunyi aiki ta hana masu karba don tyrosine kinases, kwayoyin da ke kula da abubuwan ci gaba da fibrosis. Dukkanin magungunan sun nuna cewa jinkirta cigaban IPF.
Abin takaici, mutane na iya amsawa fiye da ɗaya ko ɗaya daga cikin wadannan kwayoyi guda biyu, kuma a wannan lokacin babu hanyar da za a iya faɗa wa abin da magani zai iya zama mafi alhẽri ga mutumin. Duk da haka, gwaji mai gwadawa zai iya zama a sararin sama domin yayi la'akari da amsawar mutum akan wadannan kwayoyi guda biyu. (Ƙari a kan wannan ƙasa.)
Bugu da kari, an gane yanzu cewa mutane da yawa da IPF (har zuwa kashi 90) suna da ciwon gurasar gastroesphageal (GERD) wanda zai iya zama dan kadan cewa basu lura da shi ba. Duk da haka, "microreflux" na yau da kullum na iya zama wani abu wanda zai haifar da lalacewar lalacewa a cikin jikin huhu - kuma a cikin mutanen da ke da maganin warkaswa na warkaswa, ƙananan fibrosis zai iya haifar.
Ƙananan gwaje-gwajen da aka ƙaddara sun nuna cewa mutane da ke da IPF waɗanda aka bi da su don GERD na iya samun ci gaba mai zurfi na IPF. Duk da yake ana buƙatar da gwaji na asibiti mafi girma da kuma dogon lokaci, wasu masana sunyi imanin cewa magani "na yau da kullum" ga GERD yana da kyau a cikin mutanen da ke da IPF.
Matsaloli na Canje-canje na Nan gaba
Nazarin Halitta
An san cewa mutane da yawa da suka bunkasa IPF suna da jigilar kwayoyin halitta a wannan yanayin. Ana gudanar da bincike mai aiki don gwada alamomin alamar kwayoyin halitta a cikin al'umar al'umar al'ada ga alamar kwayoyin halitta a cikin abincin huhu na mutanen da suke da IPF. Yawancin bambance-bambance daban-daban a cikin sifofin IPF an riga an gano su. Wadannan alamomi na kwayoyin suna samar da masu bincike tare da wasu manufofi don ci gaban miyagun ƙwayoyi don magance IPF. A cikin 'yan shekarun nan, magungunan musamman "waɗanda aka tsara" su bi da IPF suna iya kaiwa mataki na gwaji.
Drugs Ana Gwada
Duk da yake muna jira don takamaiman bayani, maganin maganin miyagun ƙwayoyi, a halin yanzu an riga an gwada wa] ansu magungunan maganganu:
- Imatinib: Imatinib wani mawallacin tyrosine kinase, kamar nintendanib.
- FG-3019: Wannan miyagun ƙwayoyi wani ɓangare ne na daya wanda yake nufin haɓakar haɓakaccen nama, kuma an tsara ta don rage fibrosis.
- Thalidomide: An nuna wannan miyagun ƙwayoyi don rage ƙwayar cutar fibrosis a cikin dabbobin dabba, kuma an gwada shi a marasa lafiya tare da IPF.
Pulmospheres
Masu bincike a Jami'ar Alabama sun bayyana wani sabon fasaha inda suke tara "kwayoyin halitta" -iran da aka sanya daga jikin mutum daga jikin mutum mai suna IPF-kuma ya gabatar da kwayoyin halitta zuwa ga kwayoyin anti-IPF na nintendanib da pirfenidone. Daga wannan gwaji, sunyi imani zasu iya ƙayyade lokacin da mai yiwuwa mai yiwuwa mai yin haƙuri ya amsa da kyau ga ko dai ko duka waɗannan kwayoyi. Idan an tabbatar da kwarewa ta farko tare da ƙarin gwaji, wannan zai iya samuwa a matsayin hanyar da za a gwada gwajin gwaje-gwaje a cikin mutane da IPF.
Kalma Daga
IPF wata cuta ce mai tsanani, kuma zai iya zama yankunan don samun wannan ganewar. A gaskiya ma, mutumin da ke da IPF wanda ke yin bincike na Google a kan wannan yanayin zai iya fitowa sosai. Duk da haka, a cikin 'yan shekarun da suka wuce, an samu ci gaba sosai wajen magance IPF. An riga an amince da sababbin sababbin kwayoyi don magani, an gwada sababbin sababbin magunguna a gwaje-gwaje na asibitoci, kuma an yi bincike kan bincike don samar da sababbin maganin gaggawa.
Idan kai ko wanda kake ƙaunata tare da IPF yana da sha'awar yin la'akari da gwajin gwagwarmaya tare da daya daga cikin sababbin kwayoyi, ana iya samun bayanai game da gwajin gwaji a clinicaltrials.gov.
> Sources:
> Hershcovici T, Jha LK, Johnson T, et al. Binciken na yau da kullum: dangantakar dake tsakanin cututtuka na kwakwalwa ta tsakiya da kuma cututtuka gastro-oesophageal. Abincin Pharmacol Ther 2011; 34: 1295.
> Raghu G, Rochwerg B, Zhang Y, et al. Dokar Harkokin Kasuwanci ATS / ERS / JRS / ALAT da ke Kula da Harkokin Gudanar da Harkokin Kasuwanci: Jiyya na Fibrosis. Ɗaukakawar Dokar Neman Harkokin Gudanarwar Harkokin Gini na 2011. Am J Respir Crit Care Med 2015; 192: e3.
> Surolia R, Li FJ, Wang Z, et al. 3D Pulmospheres Serve Kamar yadda Nagartaccen Mahimmanci na Mahimmanci na Ƙira don Mahimmancin Magungunan Antifibrotic. JCI Insight 2017. 2 (2): e91377.
> Xu Y, Mizuno T, Sridharan A, et al. Rikicin RNA guda daya-ƙayyade ya nuna nau'ikan ƙwayoyin ƙwayoyin ƙwayoyin cuta a cikin Fibrosis na ƙwayar cuta. JCI Insight. 2017; 1 (20): e 90558.